Neuro Pathology Pt. 3 Flashcards
(59 cards)
A degenerative disease affecting both UMNs and LMNs with degeneration of the anterior horn cells & descending corticobulbar & corticospinal tracts
Amyotrophic Lateral Sclerosis (ALS)/ Lou Gehrig’s disease
Signs and symptoms of ALS/Lou Gehrig’s disease
- Progressive disease often leading to death in 2-5 yrs with highly variable symptoms
- Muscular weakness that spreads over time: early onset of limbs then whole body; atrophy, cramping, muscle fasciculations, or twitching (LMN signs)
- Spasticity, hyperreflexia (UMN signs)
- Sensation is preserved; sparing of bowel/bladder function; normal cognition
- Dysarthria, dysphagia, dysphonia 2ndy to pseudobulbar palsy/bulbar palsy
- Respiratory impairments
Describe the 6 stages of ALS/Lou Gehrig’s disease
- I: mild focal weakness, asymmetrical distribution; sx of hand cramping & fasciculations
- II: moderate weakness in groups of muscles, some wasting; modified independence with ADs
- III: severe weakness of specific muscles, increasing fatigue, mild-mod functional limitations, ambulatory
- IV: severe weakness & wasting of LEs, mild weakness of UEs; mod A & ADs required; wheelchair user
- V: progressive weakness with deterioration of mobility & endurance, increased fatigue, mod-severe weakness of whole limbs/trunk, spasticity, hypperrefelxia, loss of head control, max A
- VI: bedridden, dependent ADLs, FMS; progressive respiratory distress
Purpose of Riluzole in treatment of ALS
- Glutamate antagonist
- May slow progression, prolong survival especially with bulbar onset disease
- Could extend survival by 2-3 months
Weakness or paralysis of the muscles innervated by the motor nuclei of the lower brainstem; affects the muscles of the face, tongue, larynx, & pharynx
- Bulbar palsy
Bilateral dysfunction of corticobulbar innervation of brainstem nuclei; a central or UMN lesion analogous to corticospinal lesions disrupting function of anterior horn cells
- Pseudobulbar palsy
Signs and symptoms of pseudobulbar palsy
- Produces similar symptoms as bulbar palsy
- Examine for hyperactive reflexes: increased jaw jerk and snout reflex (tapping on lips produces pouting of lips)
Signs and symptoms of trigeminal neuralgia (Tic Douloureux)
- Brief paroxysms of neurogenic pain (stabbing and/or shooting pain) reoccurring frequently
- Occurs along distribution of trigeminal nerve; restricted to one side of face
- Autonomic instability: exacerbated by stress, cold; relieved by relaxation
- Light touch to face, lips, or gums will cause pain
Define Bell’s palsy
- CN VII lesion resulting in unilateral facial paralysis of both the upper and lower parts of one side of the face
- Acute inflammatory process of unknown cause resulting in compression of the nerve within the temporal bone
Signs and symptoms of Bell’s palsy
- Muscles of facial expression on one side are weakened or paralyzed
- Loss of control of salivation or lacrimation
- Acute onset commonly preceded by a day or two of pain behind the ear
- Sensation is normal
What are the 3 W’s for examining facial nerve
- CN VII
- Whistle (puff out cheeks)
- Wink
- Wrinkle (forehead)
What are the 3 peripheral nerve injury classifications
- Neurapraxia: nerve injury causing a transient & focal chemical/structural loss of function
- Axonotmesis: focal damage to the axon/myelin & varying degrees of peripheral nerve connective tissue; results in Wallerian degeneration within disrupted axons
- Neurotmesis: severance of axon/myelin & all connective tissue structures to include epineurium; complete loss of nerve function & Wallerian degeneration with no connective tissue path
What 3 neuroplasticity properties relate to the healing of a peripheral nerve injury
- Remyelination: a portion of the lost myelin can be replaced to restore neural conduction
- Axonal regeneration: axons that regenerate often do not remyelinate to their pre injury level
- Collateral sprouting: intact axons can pick up dennervated terminal targets
What are the 4 types of peripheral nerve injuries
- Mononeuropathy: involvement of a single nerve
- Mononeuropathy multiplex: involvement of 2 or more nerves without a clear pattern of polyneuropathy
- Radiculopathy: involvement of nerve root(s)
- Plexopathy: involvement of brachial or lumbosacral plexus
Signs and symptoms to look for in polyneuropathy patients indicating autonomic dysfunction
- Vasodilation and loss of vasomotor tone
- Dryness, warm skin, edema, orthostatic hypotension
Common risk factors for polyneuropathy
- Diabetes
- Renal failure
- Alcohol abuse
- Systemic autoimmune disease
- Autoimmune disease-nerve
- Nutritional imbalances
- Hereditary
- Infections
- Certain cancers
- Medications
- Toxins
- Idiopathic: ~25% of patients
Acute inflammatory demyelinating polyradiculoneuropathy presenting with rapid nonsymmetrical loss of myelin in both nerve roots & peripheral nerves
- Guillain-Barré syndrome (GBS)
Signs and symptoms of GBS
- Acute demyelination of both cranial and peripheral nerves (LMN disease)
- Sensory loss, paresthesias, pain
- Motor paresis or paralysis: relative symmetrical distribution of weakness; may produce full tetraplegia with respiratory failure
- Dysarthria, dysphagia, diplopia, & facial weakness may develop in severe cases
- Progression of days to weeks with slow recovery of ~6 months to 2 yrs
Medical management for GBS
- Plasmapheresis: therapy to remove antibodies causing clinical sx
- IVIG (intravenous immunoglobulin): immunosuppression therapy
- Analgesics: relief of pain
Slow progressive muscle weakness occurring in individuals with a confirmed history of acute polio; follows a stable period (usually 15 yrs or more) of functioning
- Postpolio Syndrome (PPS)
Signs and symptoms of postpolio syndrome (PPS)
- Gradual onset of new muscle weakness or fatigue with or without muscle atrophy or muscle/joint pain
- New symptoms >1 yr
- Abnormal fatigue: doesn’t recover easily with usual rest periods
- Myalgia, cramping, joint pain with repetitive injury, hypersensitivities
- Slow progression
- Environmental cold intolerance
- Sleep disturbances
- Decreased functional mobility, aerobic capacity, labile exercise BPs
Medical management for postpolio syndrome (PPS)
- Antidepressants: amitriptyline (Elavil), fluoxetine (Prozac)
- neurotransmitter inhibitors: decreases fatigue & sleep disorders (serotonin, norepinepherine)
Important PT considerations for postpolio syndrome
- Use whole body movements for aerobic training to avoid overworking involved muscles
- Provide discontinuous non fatiguing exercises with increased rest breaks due to fatigue
- Foster weight control/reduction programs
- Avoid muscle training for patients with severe paresis
A postsynaptic neuromuscular junction (NMJ) disorder characterized by progressive muscular weakness and fatiguability on exertion; autoimmune antibody mediated attack on acetylcholine receptors at NMJ
- Myasthenia Gravis